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cardiomyopathy pediatric

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https://www.readbyqxmd.com/read/29908011/pretransplant-increases-in-left-ventricular-volume-and-mass-are-associated-with-qt-prolongation-during-pediatric-liver-transplantation
#1
Hyun-Seok Cho, Wook-Jong Kim, Byungdoo Andrew Lee, Junki Cho, Won-Jung Shin, Gyu-Sam Hwang
Structural alterations in the cirrhotic heart may contribute to electromechanical abnormalities, represented by QT prolongation. The aim of this study was to investigate the changes in QTc according to the operative stage during pediatric LT and to identify which baseline echocardiographic parameters were associated with intraoperative QTc prolongation. Data were evaluated from 39 children undergoing LT for chronic liver disease (median age 9 months). In 19 patients (48.7%), baseline QTc was prolonged ≥440 ms (462 ± 19 ms)...
June 15, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29907873/genetic-background-of-japanese-patients-with-pediatric-hypertrophic-and-restrictive-cardiomyopathy
#2
Takeharu Hayashi, Kousuke Tanimoto, Kayoko Hirayama-Yamada, Etsuko Tsuda, Mamoru Ayusawa, Shinichi Nunoda, Akira Hosaki, Akinori Kimura
Hypertrophic cardiomyopathy (HCM) and restrictive cardiomyopathy (RCM) present a high risk for sudden cardiac death in pediatric patients. The aim of this study was to identify disease-associated genetic variants in Japanese patients with pediatric HCM and RCM. We analyzed 67 cardiomyopathy-associated genes in 46 HCM and 7 RCM patients diagnosed before 16 years of age using a next-generation sequencing system. We found that 78% of HCM and 71% of RCM patients carried disease-associated genetic variants. Disease-associated genetic variants were identified in 80% of HCM patients with a family history and in 77% of HCM patients with no apparent family history (NFH)...
June 15, 2018: Journal of Human Genetics
https://www.readbyqxmd.com/read/29900007/use-of-integrated-imaging-and-serum-biomarker-profiles-to-identify-subclinical-dysfunction-in-pediatric-cancer-patients-treated-with-anthracyclines
#3
Olga H Toro-Salazar, Ji Hyun Lee, Kia N Zellars, Paige E Perreault, Kathryn C Mason, Zhu Wang, Kan N Hor, Eileen Gillan, Caroline J Zeiss, Daniel M Gatti, Brooke T Davey, Shelby Kutty, Bruce T Liang, Francis G Spinale
Background: Anthracycline induced cardiomyopathy is a major cause of mortality and morbidity among pediatric cancer survivors. It has been postulated that oxidative stress induction and inflammation may play a role in the pathogenesis of this process. Accordingly, the present study performed an assessment of biomarker profiles and functional imaging parameters focused upon potential early determinants of anthracycline induced cardiomyopathy. Methods: Patients (10-22 years) were prospectively enrolled between January 2013 and November 2014...
2018: Cardio-Oncology
https://www.readbyqxmd.com/read/29887441/potential-for-and-timing-of-recovery-in-children-with-dilated-cardiomyopathy
#4
Matthew J Fenton, Philippa Horne, Jacob Simmonds, Sophie L Neligan, Rachel E Andrews, Michael Burch
OBJECTIVE: Understanding the clinical course and time-frame for recovery is helpful to guide management and counselling following a diagnosis of Dilated Cardiomyopathy (DCM). We aimed to document outcomes and time to recovery for a cohort of patients with a dilated cardiomyopathy phenotype. METHODS: An observational cohort methodology was used to collect retrospective data from the departmental database for those identified with DCM. Data relating to mode of presentation, echocardiographic parameters, clinical management and outcome were collated and analysed...
September 1, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29777282/ambulatory-intravenous-inotropic-support-and-or-levosimendan-in-pediatric-and-congenital-heart-failure-safety-survival-improvement-or-transplantation
#5
Sotiria C Apostolopoulou, George A Vagenakis, Alexandros Tsoutsinos, Felicia Kakava, Spyridon Rammos
End-stage heart failure (HF) frequently needs continuous inotropic support in hospital and has high morbidity and mortality in absence of heart transplantation. This study reports outcome, efficacy, and safety of continuous ambulatory inotropes (AI) and/or periodic levosimendan (LS) infusions in pediatric HF patients. The study included 27 patients, median age 9.4 (0.1-26.1) years, with severe HF (6 myocarditis, 13 dilated cardiomyopathy, 2 restrictive cardiomyopathy, 6 repaired congenital heart disease). Dobutamine and milrinone AI were administered in 21 patients through a permanent central catheter for median duration 1...
May 18, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29772353/cardiac-profile-of-chimeric-antigen-receptor-car-t-cell-therapy-in-children-a-single-institution-experience
#6
Danielle Burstein, Shannon Maude, Stephen Grupp, Heather Griffis, Joseph Rossano, Kimberly Lin
BACKGROUND: Immunotherapy with chimeric antigen receptor (CAR)-modified T-cells targeting CD19 for pediatric acute lymphoblastic leukemia (ALL) has demonstrated significant efficacy. The principle toxicity is cytokine release syndrome with resultant hypotension. However, the spectrum of cardiovascular effects associated with CAR T-cell therapy has not been systematically evaluated. METHODS: We reviewed all patients who received CD19-directed CAR T-cells at the Children's Hospital of Philadelphia between April 2012 and September 2016...
May 14, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29766225/potential-common-pathogenic-pathways-for-the-left-ventricular-noncompaction-cardiomyopathy-lvnc
#7
Ying Liu, Hanying Chen, Weinian Shou
Ventricular trabeculation and compaction are two essential morphogenetic events for generating a functionally competent ventricular wall. A significant reduction in trabeculation is usually associated with hypoplastic wall and ventricular compact zone deficiencies, which commonly leads to embryonic heart failure and early embryonic lethality. In contrast, the arrest of ventricular wall compaction (noncompaction) is believed to be causative to the left ventricular noncompaction (LVNC), a genetically heterogeneous disorder and the third most common cardiomyopathy among pediatric patients...
May 15, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29754204/diagnosing-arvc-in-pediatric-patients-applying-the-revised-task-force-criteria-importance-of-imaging-12-lead-ecg-and-genetics
#8
Michael Steinmetz, Ulrich Krause, Peter Lauerer, Frank Konietschke, Randolph Aguayo, Christian Oliver Ritter, Andreas Schuster, Joachim Lotz, Thomas Paul, Wieland Staab
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a potentially lethal disease that is well described in adults. In pediatric patients, however, identification of patients at risk of adverse events of ARVC remains a challenge. We aimed to determine which criteria of the revised Task Force Criteria (rTFC), alone or combined, have an impact on diagnosis of ARVC when compared to disease-specific genetic mutations in pediatric patients ≤ 18 years. Between September 2010 and December 2013, 48 consecutive young patients ≤ 18 years of age (mean 14, range of 12...
May 12, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29753543/increased-fracture-risk-with-furosemide-use-in-children-with-congenital-heart-disease
#9
Ji Haeng Heo, Karen L Rascati, Keila N Lopez, Brady S Moffett
OBJECTIVES: To determine the association of furosemide therapy with the incidence of bone fractures in children with congenital heart disease. STUDY DESIGN: We conducted a retrospective cohort study with data extracted from the 2008-2014 Texas Medicaid databases. Pediatric patients aged <12 years diagnosed with congenital heart disease, cardiomyopathy, or heart failure were included. Patients taking furosemide were categorized into a furosemide-adherent group (medication possession ratio of ≥70%), and a furosemide-nonadherent group (medication possession ratio of <70%)...
May 9, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29710297/pediatric-hypertrophy-cardiomyopathy-another-case-where-children-are-not-small-adults
#10
Elizabeth M McNally
No abstract text is available yet for this article.
April 18, 2018: JAMA Cardiology
https://www.readbyqxmd.com/read/29710196/long-term-outcomes-of-pediatric-onset-hypertrophic-cardiomyopathy-and-age-specific-risk-factors-for-lethal-arrhythmic-events
#11
Niccolò Maurizi, Silvia Passantino, Gaia Spaziani, Francesca Girolami, Anna Arretini, Mattia Targetti, Iva Pollini, Alessia Tomberli, Silvia Pradella, Giovanni Battista Calabri, Veronica Vinattieri, Bruno Bertaccini, Ornella Leone, Luciano De Simone, Claudio Rapezzi, Niccolò Marchionni, Franco Cecchi, Silvia Favilli, Iacopo Olivotto
Importance: Predictors of lethal arrhythmic events (LAEs) after a pediatric diagnosis of hypertrophic cardiomyopathy (HCM) are unresolved. Existing algorithms for risk stratification are limited to patients older than 16 years because of a lack of data on younger individuals. Objective: To describe the long-term outcome of pediatric-onset HCM and identify age-specific arrhythmic risk factors. Design, Setting, and Participants: This study assessed patients with pediatric-onset hypertrophic cardiomyopathy diagnosed from 1974 to 2016 in 2 national referral centers for cardiomyopathies in Florence, Italy...
April 18, 2018: JAMA Cardiology
https://www.readbyqxmd.com/read/29668027/heart-transplant-in-children-what-a-primary-care-provider-needs-to-know
#12
Swati Sehgal, Emily Shea, Lauren Kelm, Deepak Kamat
Heart transplantation is offered to children with heart failure that is not amenable to medical or surgical therapy. Indications for heart transplant include unrepairable congenital heart disease, failed palliation of congenital heart disease, and cardiomyopathies. There has been tremendous progress in this field since the first heart transplant was performed in 1967. Each year, approximately 500 pediatric heart transplants take place worldwide. Pediatric heart transplant survivors are living longer with their initial transplant...
April 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29651540/identifying-non-invasive-tools-to-distinguish-acute-myocarditis-from-dilated-cardiomyopathy-in-children
#13
Divya Suthar, Debra A Dodd, Justin Godown
There is often a diagnostic dilemma in pediatric patients presenting with depressed ventricular function, as myocarditis and dilated cardiomyopathy (DCM) of other etiologies can appear very similar. Accurate identification is critical to guide treatment and to provide families with the most accurate expectation of long-term outcomes. The objective of this study was to identify patterns of clinical presentation and to assess non-invasive measures to differentiate patients with acute myocarditis from other forms of DCM...
April 12, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29622585/prevalence-and-progression-of-late-gadolinium-enhancement-in-children-and-adolescents-with-hypertrophic-cardiomyopathy
#14
Anna Axelsson Raja, Hoshang Farhad, Anne Marie Valente, John-Paul Couce, John Lynn Jefferies, Henning Bundgaard, Kenneth Zahka, Harry Lever, Anne M Murphy, Euan Ashley, Sharlene M Day, Mark V Sherrid, Ling Shi, David A Bluemke, Charles E Canter, Steven D Colan, Carolyn Y Ho
Background -Late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMR) is believed to represent dense replacement fibrosis. It is seen in ~60% of adult patients with hypertrophic cardiomyopathy (HCM). However, the prevalence of LGE in children and adolescents with HCM is not well established. Additionally, longitudinal studies describing the development and evolution of LGE in pediatric HCM are lacking. This study assesses the prevalence, progression, and clinical correlations of LGE in children and adolescents with, or genetically predisposed to, HCM...
April 5, 2018: Circulation
https://www.readbyqxmd.com/read/29607544/outcome-of-pediatric-heart-transplantation-in-blood-culture-positive-donors-in-the-united-states
#15
Shahnawaz M Amdani, Wei Du, Sanjeev Aggarwal
Active donor infection at the time of organ procurement poses a potential infection risk and may increase post-transplant morbidity and mortality in recipients. Our hypothesis was that pediatric heart transplant recipients from blood culture positive donors (BCPD) would have increased morbidity and mortality compared to non-blood culture positive donors (NBCPD). A retrospective analysis of pediatric heart transplant recipients using the organ procurement and transplant network (OPTN) between 1987 and 2015 was conducted...
May 2018: Clinical Transplantation
https://www.readbyqxmd.com/read/29606334/apical-transverse-motion-is-associated-with-interventricular-mechanical-delay-and-decreased-left-ventricular-function-in-children-with-dilated-cardiomyopathy
#16
Wei Hui, Cameron Slorach, Mark K Friedberg
BACKGROUND: Apical transverse motion (ATM) is associated with electromechanical dyssynchrony in adult dilated cardiomyopathy (DCM). Bundle branch block electromechanical dyssynchrony is uncommon in pediatric DCM, but ATM and its association with ventricular function have not been characterized. METHODS: Fifty-six children with DCM were retrospectively studied. Using echocardiography, ATM was assessed visually and by speckle-tracking longitudinal displacement of the interventricular septal and left ventricular (LV) lateral walls in opposite directions...
March 29, 2018: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/29602613/noninvasive-echocardiographic-measures-of-pulmonary-vascular-resistance-in-children-and-young-adults-with-cardiomyopathy
#17
Dor Markush, Robert D Ross, Ronald Thomas, Sanjeev Aggarwal
BACKGROUND: Patients with cardiomyopathy (CM) are at increased risk for pulmonary hypertension (PH). Data are lacking on the use of noninvasive PH measures by echocardiography in patients with CM. The aim of this study was to evaluate the correlation between Doppler-derived echocardiographic indices and catheterization-based measurement of pulmonary vascular resistance (PVR) in children and young adults with CM. METHODS: Imaging studies were retrospectively reviewed from pediatric patients with CM who underwent both echocardiography and cardiac catheterization within a 72-hour period...
March 27, 2018: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/29587521/orthotopic-heart-transplantation-in-children
#18
Nagarajan Muthialu
Heart transplantation is a relatively infrequent but technically demanding procedure in pediatric cardiac surgery. Orthotopic heart transplantation is often offered to older children or adolescents following multiple previous operations, but with advances in intensive care, diagnostics, and management, more infants with complex congenital heart diseases are being listed for transplantation. Primary cardiomyopathies remain the most common indication. Outcomes following cardiothoracic transplantation have improved steadily in recent times...
May 2018: Asian Cardiovascular & Thoracic Annals
https://www.readbyqxmd.com/read/29581480/late-cardiovascular-morbidity-and-mortality-following-pediatric-allogeneic-hematopoietic-cell-transplantation
#19
Christine N Duncan, Ruta Brazauskas, Jiaxing Huang, Bronwen E Shaw, Navneet S Majhail, Bipin N Savani, Mary E D Flowers, Minoo Battiwalla, Kristen Beebe, Andrew C Dietz, Christopher C Dvorak, Roger Giller, David A Jacobsohn, Morris Kletzel, Paul L Martin, Eneida R Nemecek, Brandon Nuechterlein, Julie-An Talano, Michael A Pulsipher, K Scott Baker
We analyzed late cardiovascular outcomes of 661 patients who survived at least 2 years from hematopoietic cell transplantation for childhood hematologic malignancy between 1995 and 2008. Center for International Blood and Marrow Transplant Research data was supplemented with surveys focused on cardiotoxicity and potential risk factors. The median duration of follow-up was 97 months (range 24-230). 4.2% of survivors experienced at least one of the primary outcomes including coronary artery disease (0.2%), cerebrovascular accident (0...
March 26, 2018: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/29567486/genetic-evaluation-of-cardiomyopathy-a-heart-failure-society-of-america-practice-guideline
#20
Ray E Hershberger, Michael M Givertz, Carolyn Y Ho, Daniel P Judge, Paul F Kantor, Kim L McBride, Ana Morales, Matthew R G Taylor, Matteo Vatta, Stephanie M Ware
This guideline describes the approach and expertise needed for the genetic evaluation of cardiomyopathy. First published in 2009 by the Heart Failure Society of America (HFSA), the guideline has now been updated in collaboration with the American College of Medical Genetics and Genomics (ACMG). The writing group, composed of cardiologists and genetics professionals with expertise in adult and pediatric cardiomyopathy, reflects the emergence and increased clinical activity devoted to cardiovascular genetic medicine...
May 2018: Journal of Cardiac Failure
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